Abstract
The complications of Marfan syndrome (MFS), which may present as medical emergencies, are well recognised [1–3] and include aortic dissection/rupture, pneumothorax, retinal detachment, lens dislocation and joint dislocation. The optimum management of these complications has also been well described. Some of the above-mentioned complications are life-threatening, the most notorious being aortic dissection. If this condition is undiagnosed the early mortality is 1 % per each hour delay [4, 5]. The outcome is proportional to the time spent in making the diagnosis and moving the patient from the Emergency Department to the operating room. Unfortunately establishing the diagnosis of aortic dissection/rupture in the Emergency Department proves difficult, leading to avoidable mortality and morbidity. We have attempted in this chapter to highlight some of the pitfalls in the diagnosis. Our objective is that these complications will be recognised in a timely manner so that patients are moved to the appropriate facility with the least possible delay.
† Deceased
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Beighton P, editor. MKusick’s heritable disorders of connective tissue. 5th ed. St Louis: CV Mosby Company; 1993.
Pyeritz RE. The Marfan syndrome. In: Royce PM, Steinmann B, editors. Connective tissue and its heritable disorders: molecular, genetic and medical aspects. New York: Wiley-Liss; 1993. p. 437–68.
Murdoch JL, Walker BA, et al. Causes of death in the Marfan syndrome. N Engl J Med. 1972;13:804–8.
Hirst Jr AE, Johns Jr VJ, Kime Jr SW. Dissecting aneurysms of the aorta: a review of 505 cases. Medicine. 1958;37:217–79.
Anagnosopoulous CE, Manakavalan KSP, Kittle CF. Aortic dissections and dissecting aneurysms. Am J Cardiol. 1972;30:263–73.
Bourland MD. Aortic dissection. In: Rosen P, Barkin R, Braen GR, et al., editors. Emergency medicine concepts and clinical practice. 3rd ed. St Louis: CV Mosby; 1992. p. 1384.
Fielman JA. Thoracic and abdominal aortic aneurysms. In: Tintinalli JE, Krome RL, Ruiz E, editors. Emergency medicine: a comprehensive study guide. St Louis: Mosby; 1992. p. 247.
Da Sancis RW, Doroghazi RM, et al. Aortic dissection. N Engl J Med. 1987;317(17):1060–7.
Zappa MJ, Harwood-Nuss A. Recognition and management of aortic dissection and thoracic aortic aneurysm. Emerg Med Rep. 1993;14(1):3–4.
Treasure T, Raphael MJ. Investigation of suspected dissection of the thoracic aorta. Lancet. 1991;338:490–5.
Erbel R, Daniel W, Visser C, et al. Echocardiography in the diagnosis of aortic dissection. Lancet. 1989;1:457–61.
Cigarroa JE, Isselbacher EM, DeSantis RW, et al. Diagnostic imaging in the evaluation of suspected aortic dissection. N Engl J Med. 1993;328:35–43.
Morgan JM, Oldershaw PJ, Gray HH. Use of computed tomographic scanning and aortography in the diagnosis of acute aortic dissection of the thoracic aorta. Br Heart J. 1990;64:261–5.
Nienaber CA, Kodolitch Y, Nichplas V, et al. The diagnosis of thoracic dissection by non-invasive imaging procedures. N Engl J Med. 1993;328:1–9.
Panting JR, Norell MS, Baker C, Nicholson AA. Feasibility, accuracy and safety or magnetic resonance imaging in acute aortic dissection. Clin Radiol. 1995;50:455–8.
Simpson IA, De Belder MA, Camm JA, Pumphrey CW. Cardiovascular manifestations of Marfan syndrome: improved evaluation by transoesophageal echocardiography. Br Heart J. 1993;69:104–8.
Hall JR, Pyeritz RE, Dudgeon MDG, Haller A. Pneumothorax in the Marfan syndrome. Ann Thorac Surg. 1984;37:500–5.
Wood J, Bellamy D, Child A, Citron KM. Pulmonary disease in patients with Marfan syndrome. Thorax. 1984;39:780–4.
Wunsch CM, Steinmetz EF, Fisch C. Marfan syndrome and subacute bacterial endocarditis. Am J Cardiol. 1965;15:102–6.
Bowers D, Lim DW. Subacute bacterial endocarditis and Marfan syndrome. Can Med Assoc J. 1962;86:455–8.
Prevention of endocarditis. British National Formulary. No 28 and subsequent editions. London: Brtish Medical Association.
Verghese C. Anaesthesia in Marfan syndrome. Anaesthesia. 1984;39:917–22.
Wakita M, Ryu E, Nakayasu K, et al. Statistical analysis of Marfan syndrome. Acta Societatis Opthalmologicae Japonicae. 1989;93(6):682–90.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2016 Springer-Verlag London
About this chapter
Cite this chapter
Wijetunge, D.B., Child, A.H. (2016). Diagnosis and Management of Acute Complications Associated with Marfan Syndrome: Pitfalls in the Emergency Setting. In: Child, A. (eds) Diagnosis and Management of Marfan Syndrome. Springer, London. https://doi.org/10.1007/978-1-4471-5442-6_3
Download citation
DOI: https://doi.org/10.1007/978-1-4471-5442-6_3
Published:
Publisher Name: Springer, London
Print ISBN: 978-1-4471-5441-9
Online ISBN: 978-1-4471-5442-6
eBook Packages: MedicineMedicine (R0)